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Background/PurposeA small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.MethodsRetrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale.ResultsThere were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD.ConclusionMore than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment.Level of EvidenceLevel 4  相似文献   
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Even if the relationships between nutrition and inflammatory bowel disease (IBD) remain underexplored, the current literature is providing, day by day, much more evidence on the effects of various diets in both prevention and treatment of such illnesses. Wrong dietary habits, together with other environmental factors such as pollution, breastfeeding, smoke, and/or antibiotics, are among the theoretical pathogenetic causes of IBD, whose multifactorial aetiology has been already confirmed. While some of these risk factors are potentially reversible, some others cannot be avoided, and efficient treatments become necessary to prevent IBD spread or recurrence. Furthermore, the drugs currently available for treatment of such disease provide low-to-no effect against the symptoms, making the illnesses still strongly disabling. Whether nutrition and specific diets will prove to effectively interrupt the course of IBD has still to be clarified and, in this sense, further research concerning the applications of such dietary interventions is still needed.  相似文献   
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This study aimed to observe the therapeutic effects of magnesium lithospermate B on acute and chronic colitis induced by dextran sodiumsulfate (DSS) and the role of inflammasome complex (NOD-like receptor protein, NLRP; apoptosis-associated speck-like protein containing, ASC; caspase-1). Establishment of acute and chronic colitis models were by using 5% DSS oral administration in BALB/C male mice. Magnesium lithospermate B (240 mg/kg body weight) was given by subcutaneous injection. Samples were collected for biomarker assay, histological examination, immunohistochemical evaluation and western blot. There was obvious increase in TNF-α level and NLPR3, ASC, and caspase-1 expressions in acute and chronic colitis groups compared with the normal control. Significant decrease of the tumor necrosis factor-α level and the expressions of NLPR3, ASC, and caspase-1 were observed after treatment with magnesium lithospermate B. This study showed that magnesium lithospermate B could be used to treat acute and chronic colitis by inhibiting the activation of the NLRP3/ASC/Caspase-1 pathway.  相似文献   
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BackgroundManagement of inflammatory bowel diseases (IBD) in the emergency department is often suboptimal.AimsTo develop a national consensus checklist of indicators to facilitate decision-making in emergency departments concerning hospitalisation and referral for abdominopelvic computed tomography (CT).MethodsA Delphi survey was used to obtain consensus on a checklist of clinical and biological variables. 119 healthcare professionals experienced in treating IBD were invited to participate. Panellists were provided with a literature survey and invited to agree or disagree with items on a prototype checklist. Two successive rounds of voting were organised.ResultsThe prototype checklist included fifteen clinical or laboratory indicators for hospitalisation or CT. Four indicators were not retained in the Delphi process and four additional indicators added. The final indicators retained were: abdominal signs/symptoms of disease exacerbation, intravenous morphine titration, fever, vomiting, dehydration, recent intestinal surgery, ano-perineal abscess, bowel obstruction, haemodynamic instability, anaemia, acute kidney failure and elevated C-reactive protein. Consensus for the retained indicators was >88%.ConclusionsUse of this consensus checklist for the management of IBD in the emergency department may help improve standards of care and thus reduce the burden of these diseases.  相似文献   
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目的探讨化浊解毒方治疗溃疡性结肠炎(UC)的治疗作用及机制。方法120例UC患者按照随机数字表法分为观察组、对照组各60例。观察组予中药化浊解毒方口服,每日1剂,早晚2次温服;对照组予美沙拉嗪肠溶片口服,1.0 g/次,3次/d。2组疗程均4周。对比2组治疗前后Geboes指数、结肠镜下黏膜表现、生活质量评分、疾病活动指数及血清炎性因子IL-8、IL-35水平,凝血指标血清FIB水平,统计治疗后1年内复发情况。结果治疗后,观察组Geboes指数、疾病活动指数及血清炎性因子IL-8水平、凝血指标血清FIB水平均较本组治疗前降低,生活质量评分、血清炎性因子IL-35水平升高(P<0.05);且观察组治疗后疾病活动指数及血清炎性因子IL-8水平、凝血指标血清FIB水平均低于对照组,生活质量评分、血清炎性因子IL-35水平均高于对照组(P<0.05)。与对照组相比,治疗组糜烂、溃疡改善不明显(P>0.05),充血水肿、颗粒样变等肠镜表现改善情况均优于对照组(P<0.05)。治疗结束1年观察组复发率为10.64%,对照组为23.53%,2组差异有统计学意义(P<0.05)。结论化浊解毒方能改善UC患者临床症状,修复肠黏膜病理损伤,降低复发率;其机制可能与调节血清炎性因子IL-8、IL-35和凝血因子FIB水平有关。  相似文献   
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Collagenous colitis is characterized by the presence of a thick subepithelial collagen band in the colonic mucosa. The condition was diagnosed on rectal biopsy in 10 patients (one male, nine females) who presented with watery diarrhoea. Although rectal mucosal erythema was present in three and ulceration in two, the mucosa was of normal endoscopic appearance in five of the patients. There was marked variability in the thickness of the submucosal collagen band, both between and within individuals. Empirical drug therapy included sulphasalazine, glucocorticoids and antidiarrhoeals. All patients reported symptomatic improvement.  相似文献   
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褪黑素对结肠黏膜上皮细胞氧化应激的影响   总被引:3,自引:1,他引:2  
目的 探讨褪黑素对大鼠结肠黏膜上皮细胞氧化应激的影响。方法 在分离培养正常大鼠结肠黏膜上皮细胞基础上,利用FeSO4 H2O2产生*OH攻击制备结肠黏膜细胞氧化损伤模型,同时预先加入褪黑素并观察损伤减轻程度。实验设正常对照组、模型对照组、5-氨基水杨酸给药组(0.1mmol/L),褪黑素给药组(0.01、0.1、l.0mmol/L)。培养一定时间后,取上清测乳酸脱氢酶(LDH)、丙二醛(MDA)、谷胱甘肽过氧化物酶(GSHPx)、过氧化氢酶(CAT)、超氧化物歧化酶(SOD)、一氧化氮(NO)和黏液含量。结果 模型组大鼠结肠黏膜上皮细胞:MDA、LDH和NO水平增高,CAT、GSHPX、SOD和黏液含量减少。不同剂量褪黑素呈剂量依赖性的减少LDH释放,抑制MDA和NO的形成,恢复黏液、CAT、GSHPX及SOD水平。结论 褪黑素对大鼠结肠黏膜氧化损伤具有保护作用。  相似文献   
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